1. Esophageal Motility Disorders

2. Esophageal Disorders Motility Anatomic & Structural Reflux Infectious
Neoplastic
Miscellaneous

3. Esophageal Anatomy Upper Esophageal Sphincter (UES)
Esophageal Body (cervical & thoracic)
18 to 24 cm
Functionally the esophagus can be considered in three zones (UES, body, LES). The upper esophageal sphincter (UES) is tonically closed and contracts during inspiration, preventing air from entering into the gastrointestinal tract, and reflux. It relaxes during swallow, belching and vomiting. The lower esophageal sphincter (LES) maintains a steady baseline tone (~20 mm Hg) to prevent gastric contents from entering the esophagus. The LES also contracts during periods of increased intra-abdominal pressure, preventing reflux due to the pressure build up in the abdomen. The esophagus has 2 muscle layers: the inner circular layer and the outer longitudinal layer. The longitudinal muscle shortens the esophagus, while the circular muscle forms lumen-occluding ring contractions. The combination of these localized contractions is responsible for peristalsis. The proximal esophagus contains striated muscle and the distal esophagus smooth muscle, with a long transition zone between. Striated muscle is “faster”.
Lower Esophageal Sphincter (LES)

4. Normal Phases of Swallowing
Voluntary
oropharyngeal phase – bolus is voluntarily moved into the pharynx
Involuntary
UES relaxation
peristalsis (aboral movement)
LES relaxation
Between swallows
UES prevents air entering the esophagus during inspiration and prevents esophagopharyngeal reflux
LES prevents gastroesophageal reflux
peristaltic and non-peristaltic contractions in response to stimuli
capacity for retrograde movement (belch, vomiting) and decompression
Primary peristalsis is a wave that strips the esophagus proximal to distal, pushing the food bolus aborally toward the stomach. In the body, the wave travels at 3-4 cm/s. Secondary peristalsis is induced by esophageal distension from retained bolus, reflux, or swallowed air. It’s role is to clear the esophagus of retained food. Tertiary contractions are simultaneous (nonperistaltic), dysfunctional contractions and have no known physiologic role and are seen more often in the elderly and can be induced by stimuli (stress, reflux, etc.).

5. Normal Swallowing Cortical Swallowing Areas Swallowing Center
Frontal cortex
Swallowing Center
Brainstem
Motor Nuclei
Primary peristalsis is triggered by the swallowing center. Myogenic propagation occurs in the body of the esophagus.
Oropharynx & Esophagus

6. Motility Disorders upper esophageal esophageal body LES
UES disorders
neuromuscular disorders
esophageal body
achalasia
diffuse esophageal spasm
nutcracker esophagus
nonspecific esophageal dysmotility
LES
hypertensive LES
primary disorders
achalasia
diffuse esophageal spasm
nutcracker esophagus
nonspecific esophageal dysmotility
secondary disorders
severe esophagitis
scleroderma
diabetes
Parkinson’s
stroke
These can be further divided into hypomotlity and hypermotlity disorders.

7. Motility Disorders diagnostic tools
cineradiology or videofluoroscopy (MBS)
barium esophagram
esophageal manometry
endoscopy

8. Normal Manometry
From S&F, Notice transition zone (mid-esophagus) with smaller amplitude contractions.

9. Upper Esophageal Motility Disorders
cause oropharyngeal dysphagia (transfer dysphagia)
patients complain of difficulty swallowing
tracheal aspiration may cause symptoms
pharyngoesophageal neuromuscular disorders
stroke
Parkinson’s
poliomyelitis
ALS
multiple sclerosis
diabetes
myasthenia gravis
dermatomyositis and polymyositis
upper esophageal sphincter (cricopharyngeal) dysfunction
These disorders tend to be considered separately from disorders of the body and LES because they are usually due to problems with striated muscle or its extrinsic innervation.

10. UES Disorders cricopharyngeal hypertension cricopharyngeal achalasia
elevated UES resting tone
poorly understood (reflex due to acid reflux or distension)
cricopharyngeal achalasia
incomplete UES relaxation during swallow
may be related to Zenker’s diverticula in some patients
clinical manifestations
localizes as upper (cervical) dysphagia
within seconds of swallowing
coughing, choking, immediate regurgitation, or nasal regurgitation
diagnosis: swallow evaluation & modified barium swallow

11. Motility Disorders of the Body & LES
symptoms: usually dysphagia (intermittent and occurring with liquids & solids)
diagnostic tests
barium esophagram
endoscopy
esophageal manometry
disorders
achalasia
diffuse esophageal spasm (DES)
nutcracker esophagus
hypertensive LES
nonspecific esophageal dysmotility
hypomotility
hypermotlity

12. Achalasia first clinically recognized esophageal motility disorder
described in 1672, treated with whale bone bougie
term coined in 1929
dual disorder
LES fails to appropriately relax
resistance to flow into stomach
not spasm of LES but an increased basal LES pressure often seen (55-90%)
loss of peristalsis in distal 2/3 esophagus
Achalasia means “failure to relax.” Incomplete LES relaxation without aperistalsis can be an early manifestation of achalasia, but infrequently.

13. Achalasia epidemiology pathology 1-2 per 200,000 population
usually presents between ages 25 to 60
male=female
Caucasians > others
average symptom duration at diagnosis: 2-5 years
pathology
loss of ganglionic cells in the myenteric plexus (distal to proximal)
vagal fiber degeneration
underlying cause: unknown
autoimmune? (antibodies to myenteric neurons in 50% of patients)

14. Achalasia clinical presentation diagnosis
solid dysphagia % (75% also with dysphagia to liquids)
post-prandial regurgitation 60-90%
chest pain 33-50%
pyrosis 25-45%
weight loss
nocturnal cough and recurrent aspiration
diagnosis
plain film (air-fluid level, wide mediastinum, absent gastric bubble, pulmonary infiltrates)
barium esophagram (dilated esophagus with taper at LES)
good screening test (95% accurate)
endoscopy (rule out GE junction tumors, esp. age>60)
esophageal manometry (absent peristalsis,  LES relaxation, & resting LES >45 mmHg)

15. Achalasia treatment - reduce LES pressure and increase emptying
nitrates and calcium channel blockers
50-70% initial response; <50% at 1 year
limitations: tachyphylaxis and side-effects
botulinum toxin (prevents ACH release at NM junction)
90% initial response; 60% at 1 year
pneumatic dilation (disrupt circular muscle)
60-95% initial success; 60% at 5 years
recent series suggest 20-40% will require re-dilation
risk of perforation 1-13% (usually 3-5%); death %
surgical myotomy (open or minimally-invasive)
>90% initial response; 85% at 10 years; 70% at 20 years (85% at 5 years with min. inv. techniques)
<1% mortality; <10% major morbidity
10-25% acutely develop reflux, up to 52% develop late reflux
Medical therapy usually reserved for individuals unwilling or unable to have more definitive therapy.

16. Spastic Motility Disorders of the Esophagus
“lumper” approach
normal
achalasia
spastic motility disorder
“splitter” approach (radiology and manometry)
diffuse esophageal spasm
nutcracker esophagus
hypertensive LES
nonspecific esophageal dysmotility
“splitting” has not resulted in a clinical benefit
In studies, all 4 have similar presenting sxs, the pathogenesis of all 4 remains unknown (maybe the same), treatment approaches are similar for all 4, and outcomes do not vary with subtype. In all of these, its hard to show that sx improvement correlates to manometric improvement. In fact, you can find asx and symptomatic patients with the exact same motility patterns. Unlike achalasia, these conditions do not progress. Neither the history nor manometric findings are accurate enough to confirm an esophageal cause for chest pain.

17. Diffuse Esophageal Spasm
frequent non-peristaltic contractions
simultaneous onset (or too rapid propagation) of contractions in two or more recording leads
occur with >30% of wet swallows (up to 10% may be seen in “normals”)
This condition is often split from the rest because the response to swallows is non-peristaltic.

18. Nutcracker Esophagus high pressure peristaltic contractions
avg pressure in 10 wet swallows is >180 mm Hg
33% have long duration contractions (>6 sec)
may inter-convert with DES

19. Nonspecific Esophageal Dysmotility
Hypertensive LES
Nonspecific Esophageal Dysmotility
high LES pressure
>45 mm Hg
normal peristalsis
often overlaps with other motility disorders
abnormal motility pattern
fits in no other category
non-peristalsis in 20-30% of wet swallows
low pressure waves (<30 mm Hg)
prolonged contractions
1/3 of pts with non-specific dysmotility will have LES hypertension.

20. Spastic Motility Disorders of the Esophagus
epidemiology
any age (mean age 40)
female > male
symptoms
dysphagia to solids and liquids
intermittent and non-progressive
present in 30-60%, more prevalent in DES (in most studies)
chest pain
constant % across the different disorders (80-90%)
swallowing is not necessarily impaired
can mimic cardiac chest pain
pyrosis (20%) and IBS symptoms (>50%)
symptoms and manometry correlate poorly
Pts often present with both sxs. Rare to see weight loss due to dysphagia here.

21. Spastic Motility Disorders of the Esophagus
diagnosis
manometry
barium esophagram
endoscopy
pH monitoring
treatment
reassurance
nitrates, anticholinergics, hydralazine - all unproven
calcium channel blockers - too few data with negative controlled studies in chest pain
psychotropic drugs – trazodone, imipramine and setraline effective in controlled studies
dilation - anecdotal reports, probable placebo effect
Some anecdotal evidence that the drugs can improve manometric findings, but clinical trials have been disappointing.
In the trazodone ( mg/d) study, a significant improvement was seen but it was not dependent on manometric improvement.
A JAMA trial of dilation found a positive effect equaled by a placebo dilator.

22. Manometry in Esophageal Symptoms
Non-Cardiac Chest Pain
Dysphagia
JE Richter, Ann Int Med, 1987

23. Hypomotilty Disorders
primary (idiopathic)
aging produces gradual decrease in contraction strength
reflux patients have varying degrees of hypomotility
more common in patients with atypical reflux symptoms
usually persists after reflux therapy
defined as
low contraction wave pressures (<30 mm Hg)
incomplete peristalsis in 30% or > of wet swallows

24. Hypomotilty Disorders
secondary
scleroderma
in >75% of patients
progressive, resulting in aperistalsis in smooth-muscle region
incompetent LES with reflux
other “connective tissue diseases”
CREST
polymyositis & dermatomyositis
diabetes
60% with neuropathy have abnormal motility on testing (most asx)
other
hypothyroidism, alcoholism, amyloidosis

25. Nonischemic Chest Pain
remains poorly understood (functional chest pain)
enthusiastic investigation finds numerous associations in studies
psychiatric disorders (depression, panic or anxiety disorder…)
esophageal disorders (GERD, motility disorders…)
musculoskeletal disorders
cardiac disease (microvascular, MVP, tachyarrhythmias…)
GERD is by far the most common, diagnosable, esophageal cause
50-60% of patients have heartburn or acid regurgitation symptoms
50% have abnormal esophageal pH studies (not always correlating to sxs)
very low incidence of endoscopic findings
“PPI Test” may be best and most cost-effective approach
a small subset of patients with non-GERD NCCP display a variety of esophageal motility disorders
symptoms and motility findings correlate poorly
esophageal hypersensitivity/hyperalgesia may explain the symptoms